Transcript Palliative Care in the Pediatric CVICU

Palliative Care in the
Pediatric CVICU
Case Studies
Priya Bhat, MD, MS
Pediatric Palliative Care Conference
May 2, 2012
Outline
Introduction
Pediatric death
“A Good Death”
Palliative Care defined
Case 1: Isaiah
Prenatal and pre-operative
counseling
Medical education
Case 2: Sara
Healthcare costs
Compassion fatigue
Case 3: Ethan
Parental perceptions
Introduction
~54,000 children die annually in the US.
~7 million children worldwide could benefit from
palliative care; 5000-8600 per day in US!
Infant mortality rate in US = ~7 per 1000 live
births.
Death rate among children <1yo is higher than
that in any other pediatric age group.
Most common etiology is congenital heart disease
(CHD).
Most children die in the hospital, particularly the
ICU.
>90% of these spend their entire life in the hospital.
A “good” death?
“A decent or good death is one that is: free
from avoidable distress and suffering for
patients, families, and caregivers; in
general accord with patients’ and families’
wishes; and reasonably consistent with
clinical, cultural, and ethical standards.”
Institute of Medicine. Approaching Death: Improving Care at the End of Life. 1997.
“Death is rarely good, but a constellation of factors add up to
a better death.”
Gazelle G. A good death: Not just an abstract concept. J of Clin Onc. 2003.
“A good death is hard to define, however, for a good death is
as complex as the good life of which it is an extension.”
Kahn D, Steeves R. Narrative of dying aimed at understanding a ‘good death.’ Textbook of Palliative
Nursing. 2001.
WHO Definition of PPC
“…aims to improve the quality of life of
patients facing life-threatening illness, and
their families, through the prevention and
relief of suffering by early identification and
treatment of pain and other problems,
whether physical, psychosocial, or spiritual.”
Total care of child’s mind, body, spirit + family support.
Begins when illness is diagnosed, and continues irrespective of
whether or not child receives disease-directed treatment.
Emphasis on alleviating child’s physical, psychological and
social distress.
Broad multidisciplinary approach including family and available
community resources.
Can be provided in tertiary-care facilities, community health
centers, home.
PPC in the CVICU
Prenatal diagnosis
~75% of all hypoplastic left heart syndrome
(HLHS) cases diagnosed prenatally.
Atz et al. JTCVS. 2010.
Curative vs. palliative surgery; multiple
operations.
Promising short-term survival vs. uncertain
long-term prognosis.
Considerable variation in mortality and
morbidity among institutions.
Cardiac transplantation.
High medical costs.
Surgical vs. medical management
philosophies.
Case 1
Isaiah’s African-American parents presented to an
outside ER intoxicated. Mom was in labor and had
had limited prenatal care during her pregnancy.
Mom is 25 years old; this is her 4th living child and 6th
pregnancy. She admitted to marijuana use during
pregnancy. Dad is 19 years old, lives with GM.
Delivery was reportedly uncomplicated but the infant’s
BAL was 0.17. He had facies characteristic of fetal
alcohol syndrome. Birth weight was 2.0 kg.
Within the first 24 hrs of life, the infant developed
respiratory distress and cyanosis. He was intubated.
At AHMC NICU, a diagnosis of HLHS was made.
Prostaglandin infusion was started.
Hypoplastic Left Heart Syndrome
Surgical Options for HLHS- Stg 1
Norwood Operation with modified BT shunt
(or Sano Modification)
Hybrid Stage 1- PDA stent, bilateral PA
bands, atrial septostomy
Diagnosis List
Hypoplastic Left Heart
Syndrome
Severe tricuspid regurgitation
Fetal Alcohol Syndrome
Small for gestational age
Eventration of right diaphragm
Hypoplastic right lung (20-25%
decreased volume)
Bilateral cryptorchidism
Parental alcohol addiction
Possible FAS in siblings
Poor social situation
Other family members/caretakers
with alcoholism
Case 1
NICU/Cardiology teams face massive
denial from mother regarding diagnoses.
Isaiah is stable but requiring increasing
ventilator support.
Staffing is arranged on DOL 11.
Mother is intoxicated in court the day prior
and is subsequently hospitalized for
substance abuse.
Custody of other 3 children given to MGM;
parents retain medical decision-making
rights.
Staffing is held with NICU and Cardiology
teams, nursing and Dad.
Treatment options for HLHS
1. Palliative surgery (Norwood vs. hybrid stage
I, Stage II- Glenn vs. hemi-Fontan, Stage IIIFontan).
5-yr survival = 38-72%.
2. Cardiac transplantation
5-yr survival = 55-76%; however, if waitlist
mortality is included, survival = 54%.
3. Palliative care*
Bidirectional Glenn
Fontan
Palliative Care for HLHS
2008 data from England, Australia:
Similar surgical outcomes as US.
Parents counseled about availability of
PPC; surgery is recommended.
Termination of pregnancy after prenatal
dx of HLHS: ~20% of cases in US.
- Allen et al. J Ultrasound Med, 2005.
Doctrine of informed consent:
1. Recommended treatment option,
2. All reasonable alternatives,
3. Consequences of not choosing lifeprolonging therapy.
The PPC Debate
Con:
Outcomes for HLHS surgery are good, and
similar to those for other CHD and critical
pediatric illnesses.
Therefore, palliative care is not an acceptable
alternative and should not be offered.
Pro:
As many parents and physicians would choose
palliative care, it is a reasonable option
regardless of surgical outcomes.
Many factors other than survival sway parental
decisions.
No outcome statistic can override the
reasonable person standard; therefore,
palliative care is a reasonable treatment option.
Parental Counseling for HLHS
Prsa et al, Pediatrics, 2010: Survey of 749 pediatric cardiologists
and surgeons.
When counseling parents of newborns with HLHS,
99.7% discussed staged palliative surgery,
67% discussed cardiac transplantation,
62.2% discussed compassionate care without surgery.
Only 14.9% discussed all options; 76.2% recommended surgery
over other options.
When counseling parents after prenatal dx of HLHS,
98.8% discussed continuation of pregnancy + palliative surgery,
53.5% discussed continuation of pregnancy + cardiac
transplantation,
56.9% discussed continuation of pregnancy + palliative care,
74.3% discussed termination of pregnancy.
36.5% discussed all options; 56% recommended surgery over other
options.
What would YOU do?
Prsa et al. Pediatrics, 2010.
Post-natal dx of HLHS:
Staged palliative surgery= 42%
Cardiac transplantation= 2%
Palliative care= 19%
Prenatal dx of HLHS:
Continuation of pregnancy + surgery= 24%
Continuation of pregnancy + transplantation= <1%
Termination of pregnancy or continuation or
pregnancy + palliative care= 47%.
Kon et al. Am J of Cardiol, 2003: 215 neonatologists,
intensivists, cardiologists, surgeons.
Post-natal dx: 32%- surgery, 6%- transplant/bridge to transplant, 28%
palliative care.
Prenatal dx: 22%- continue pregnancy, 48%- termination.
~55% of parents of HLHS children opt for
palliative care when presented with all
treatment options.* (*Data from 2000, 2001).
Case 1
Given choice of palliative care vs.
Norwood operation, dad chose surgery.
On DOL 16, Isaiah had long and
complicated operation but was able to
separate from cardiopulmonary bypass
for ~2hrs.
During diaphragm repair, he developed
pulmonary hemorrhage, severe
ventilatory difficulty, hypoxia.
After 2-3 hours of consideration,
decision made to transition to ECMO.
Extracorporeal Membrane Oxygenation
Case 1
Returned to PSHU coagulopathic, anuric.
ECMO flow limited by small cannula size.
Dad present at bedside daily. Mom present
since POD #1.
On POD #3, PRISMA initiated.
Disagreement among healthcare staff.
Mom initially did not want to continue.
Ultimately chose PRISMA x 48hrs.
Case 1
Later that day, worsening lactic
acidosis, progressive coagulopathy,
capillary leak, shedding of bowel
mucosa, GI bleeding.
Head US shows early evidence of
ischemic injury; patient has not woken
up since OR.
Lactate>14 all night.
Case 1
Parents independently feel like Isaiah
is suffering, dying, that “it was time.”
Deny offers for family support, chaplain.
Request immediate withdrawal of
support.
Father holds baby while supported on
ECMO and extubated.
Isaiah is placed on bed, circuit
clamped. Expires peacefully 15
minutes later with parents holding his
hands.
Palliative Care Education
Hilden et al. J Clin Oncol, 2001: 228 pediatric
oncologists in USA, Canada, UK
92% learned PPC through ‘trial and error.’
38% had associated traumatic experience with pt.
1970s: 6% of medical schools incorporated palliative
care in curriculum; 1990s: 30% (mostly pre-clinical or
elective).
Typical 1st exposure is during residency.
Regardless of level, method, or type of educational
intervention or evaluation, improvement is seen in outcome
measures.
Barriers to PPC education = lack of time, lack of
knowledgeable faculty and mentors.
Jan 2010: 74 active palliative care fellowships.
Role for specially trained, board-certified palliative care NPs.
Case 2
Sara is a 4mo female infant with prenatally
suspected chromosomal trisomy and
unbalanced AV septal defect.
Mom is 39yo, dad is 42yo; middle-class,
Hispanic, Catholic.
Due to religious beliefs, they choose to
continue pregnancy to term.
Upon delivery, she is diagnosed with
trisomy 21 (Down Syndrome), UAVSD (Rdominant), coarctation of aorta. Parents
request full medical and surgical treatment.
Unbalanced atrioventricular septal defect
Diagnosis List
Unbalanced AV septal defect
Right-ventricle dominant
Bilateral superior venae cavae
Single ventricle
Coarctation of the aorta
Trisomy 21 (Down Syndrome)
Bilateral clubbed feet
Cystic right kidney
Tracheomalacia
Case 2
Sara undergoes neonatal coarctation
repair, followed by pulmonary artery band
placement a month later.
She is discharged home where she grows,
develops and returns at age 4 months for
her bilateral bidirectional Glenn and
Damus-Kaye-Stansel operations.
Her operative course is complicated by 3
CPB runs for hypoxemia, bradycardia,
ventricular dysfunction.
She is returned to the PSHU with an open
sternum with snares in place for possible
ECMO cannulation.
Case 2
Cardiac arrest, followed by E-CPR ensues.
Sara develops severe capillary leak; Glenn
pressures are noted to be elevated.
Serial head US demonstrate evidence of
hypoxic ischemic injury.
10 days after initial surgery, she undergoes
takedown of her Glenn and placement of
central shunt. She is taken off ECMO
successfully.
She remains an ECMO candidate.
Parents kept updated regularly and
continue to request full support.
Case 2
Sara’s condition continues to deteriorate
over the next month with: capillary leak,
arrhythmia, thrombocytopenia, copious
chest tube output (despite thoracic duct
ligation and chemical pleurodesis), diastolic
dysfunction, kidney failure.
She has another brief cardiac arrest. Head
US demonstrates evolving global ischemic
injury.
Parents are informed of her grim condition
but struggle with their choices. She
remains full code.
The medical team decides that she is no
longer an ECMO candidate.
Economic$ of $ingle ventricle $urgery
Between 1998-2007, cumulative median length of
stay, costs and charges for 3-stage palliative surgery:
LOS: 44 days
Costs: $171,672
Charges: $376,403
Increasing costs thought to be due to increasing
survival: 57%82%.
Cardiac transplantation associated with highest
resource utilization and costs.
Highest cost for palliative surgery followed by transplantation.
26-36% likelihood of death or transplantation following stage I
surgery.
Costs comparable to other major congenital
malformations, prematurity…?
Appropriateness of use of limited societal resources.
Dean et al. Pediatrics, 2011
Case 2
Sara has 4 cardiac arrests in 24 hours,
many more over following month.
Massive work-up initiated: EEG, head/abd
US, specialized blood tests.
Dialysis is started and poorly tolerated.
Anasarca worsens. Tremendous skin
breakdown with weeping and blisters
occurs.
Medical staff struggles with caring for infant
because of difficulty with pain control.
Parents want Sara to “make the decision.”
Somewhat mixed messages from medical
team. She remains full code.
Case 2
“What are we doing?”
Sara becomes septic. She has cardiac
arrests nearly every day, many of which are
witnessed by her family.
Inotropic support is drastically escalated.
During her final cardiac arrest, MD suggests
that mom hold her. She passes away.
Parents seem stunned, distraught.
Medical staff are supportive but drained.
Compassion Fatigue
Described in 1992 when studying burnout in
ER nurses: “A unique form of burnout that
affects people in caregiving professions.”
Sx: Fatigue, avoidance, depression,
overeating, headaches, bodyaches,
cynicism, boredom, loss of compassion,
poor communication, more sick days,
decreased productivity.
Aggravated by high workloads, staff
shortages.
Disenfranchised grief; ongoing fluctuation
between focusing on loss (experiencing
grief) or repressing it (avoidance).
Need for greater support for healthcare
professionals.
Case 3
Ethan is a Caucasian male neonate
with prenatally diagnosed HLHS.
Post-natally, he is noted to have
multiple congenital anomalies.
32yo mother, 38yo father, 5yo sister,
17yo step-brother. Middle-class,
employed.
Palliative care never offered.
Stage I surgery performed on DOL 11.
Diagnosis List
Hypoplastic left heart syndrome
Coffin-Siris syndrome
Absent corpus callosum,
coprocephaly.
Abnormal gut anatomy, delayed
gastric emptying, pyloric hypertrophy
Unilateral choanal atresia
Case 3
First extubation attempt occurs 3 weeks
post-op followed by multiple failed attempts
and 3 cardiac arrests over next 3 mos.
He is treated for necrotizing enterocolitis,
has significant feeding difficulties,
undergoes gastrostomy, jejunostomy.
Successfully extubated 3 mos after initial
surgery.
Over next 2 mos, has more feeding
difficulties, failure to thrive.
Case 3
At 6 mos age, 3.5-kg, undergoes
bidirectional Glenn, TV plasty.
Fails extubation repeatedly. Has multiple
cardiac arrests.
Develops junctional rhythm requiring atrial
pacing via temporary pacing wires, which
he pulls out.
Access is difficult because of vessel
occlusion; however, indwelling catheters
have to be removed because of infections.
Undergoes Nissen, pyloroplasty at 7 mos.
Ethan’s R diaphragm is plicated; new
invasive lines are placed.
Case 3
Ethan tolerates feeds but fails to gain
weight.
He fails extubation repeatedly;
bronchoscopy demonstrates airway
pathology.
At 8 mos of age, tracheostomy is
discussed with parents.
Cardiac cath demonstrates elevated
Glenn pressures, collateral vessels.
Case 3
Ethan’s Glenn is taken down at 9 mos age,
6-mm central shunt and new temporary
pacing wires placed.
Develops slow junctional rhythm and
evidence of pacemaker dependence.
Fails extubation repeatedly. Tracheostomy
at 11 mos. Transitions to trach collar.
Starts having intractable seizures, requires 3
anticonvulsants for control.
Head CT scan shows diffuse hypoxic
ischemic injury.
“When our son…”
Case 3
Ethan celebrates 1st birthday in PSHU.
Mom researches and suggests switching
him to ketogenic formula.
After discussion with Neurology, Ketocal is
started.
He finally starts gaining weight.
He develops complete pacemaker
dependence; when temporary wires
malfunction, he rapidly decompensates.
When presented with need for PPM,
parents hesitate. Option of palliative care is
presented.
“The staff was…”
Parental Perceptions
Importance of effective communication:
Honest, complete information.
Delivered in timely, compassionate manner.
Understandable language.
Availability of medical staff for discussions.
Conflicting information, “too many cooks in the
kitchen” = mistrust.
Displaying empathy, emotion = support.
Sensitivity, interpersonal skills felt to be more important
than professional status.
Loss of control
Involvement in decision-making.
Spirituality and religion.
Prayer, access to clergy, faith.
Connection to child.
Case 3
2 weeks later, a staffing is held. Parents
request LET (limited end-of-life therapy)
orders.
A week later, parents disclose that they
would like to discontinue pacing soon.
They request comfort care in the interim,
minimal blood draws, positive energy,
respect of their privacy.
Ethan develops respiratory illness; parents
consent to work-up, antibiotics.
Though initially hesitant, his parents
consent to transfer to Step-down Unit.
“After exhausting every…”
Parental Perceptions
Transcendent quality of parent-child
relationship.
Value of parental contribution to child’s care.
Importance of time alone with child.
Evaluation of meaning of loss.
Altruism
Organ donation.
Relief of external signs of pain, suffering.
Continuation of bereavement support after
death.
Sense of community within hospital.
MDs, nurses often considered more involved during dying
process than other family, friends.
Case 3
On the selected day, at nearly 14 mos age,
Ethan is transferred to a private room.
Feeds and medications are discontinued.
Fentanyl and versed infusions are started for
comfort.
Family and medical staff are invited to say
their goodbyes.
That evening, per his parents’ wishes, his
pacemaker was stopped and wires were cut.
“On Ethan’s final day…”
“Anyone can slay a dragon. It takes a real hero to wake up every
day and learn to love the world all over again.”